Tag Archives: marfans

This post is brought to you by a cocktail of painkillers, hypnotics, and anxiolytics, administered by a lovely NHS anaesthetist whose name I can’t remember – only the sight of her leaning over me with a smile: “You’re going to forget my name.”

Just some routine Marfans stuff. But two days later, I’m still swimming through dreams. I like to think the lingering hypnotics are giving me the authentic Rossetti experience, minus the raccoon hiding in the wardrobe. And the genius.

Hanging onto the tail of this wooziness, I want to talk about childhood, but also about Jan Švankmajer’s gorgeously surreal 1988 film, Alice: “A film for children… perhaps.”

Years ago, a counsellor complimented my socks. They were pale blue and white striped, up to the knee, and they were perfect, she said, “because you’re Alice in Wonderland.”

This probably wasn’t wise of her.

Like a lot of only children travelling with military parents, I was prone to dissociation. That’s the psychology term for intense daydreaming. And I mean intense – to the point that reality was almost entirely blocked out for the majority of every waking day. Mostly, it was paradise, but there were snags. Teachers found they had to full-on yell to catch my attention. I once kicked a cannonball-sized hole through a porter cabin wall without noticing.

Dissociation is a creative coping mechanism for when life is unstable or lonely. It’s very much like the intravenous sedative experience – a protective measure that picks you up and whisks you away. Eventually, children who habitually dissociate grow up to remember more about their dream worlds than the reality of the past. Some of them become writers. Ahem.

Onto Alice. As a chronically fantastical child, I was rankled by the Disney version of Alice In Wonderland. It was all so very pastel, so very clean. Nothing pristine, I knew, could ever be magical. Childhood is frightening, and nonsensical, and inappropriately hilarious – much like the original Wonderland tales.

Children inhabit another planet. Lewis Carol knew that.

So on the other hand, you have Jan Švankmajer’s Alice. A film for children… but probably not for parents. The White Rabbit is taxidermy, leaking sawdust. Socks have teeth and the jam is full of drawing pins. Wonderland itself is a wreck of broken china and splinters, pickled specimens, potted tongue (prone to slithering), and other Victorian relics. Alice – the only living person in the film – has a way of turning into a decidedly spooky china doll and back again, tearing off the chrysalis of painted skin and making a run for it. Like the doll, her facial expression never changes. She never smiles or frowns. Those are things you do for others, and this Alice is content to be alone with her imagination.

Drowsy and doped as I am, it strikes me how strangely authentic Švankmajer’s vision of childhood imagination was.

In 1989, the year after Švankmajer released Alice, I was three. The Navy posted Dad to Scotland, and we ended up living on a dismal estate near a submarine base. Someone had spray-painted a peace symbol on the garden fence for our arrival. I wasn’t ready for school, and we never stayed anywhere long enough to make friends. I think I tried, on a few sparse occasions, but it was so much effort for so little return when inside was infinitely better than outside.

Perhaps it was the jerky stop-motion animation, or the twisted quality – lurking on the indistinct border between dreams and nightmares – but watching Švankmajer’s Alice for the first time a few weeks ago, I began to spontaneously remember scraps of day-to-day surrealism from that time in Scotland.

Someone – I forget who – told me bubbles were living things, and that when you popped them, they died.

There was a nearby play park where a boy knocked himself out trying to make the swings turn full circle. I saw his body in a red tracksuit, face down on the ground. ‘Cracked his head open’, I thought for years, was literal. Bash the hinge hard enough and the skull pops open like a spring-loaded box.

That play park was an island of concrete amongst hillocks of unkempt greenery. One day, some other children took my doll, so I wandered off to where you could see the Forth Rail Bridge, muddy-bloody coloured, in the distance. Over a hill, I found the skull of a ram, picked clean. The coiled horns looked like the fossils in my dinosaur books. I can’t recall the doll.

From the spare bedroom, if the light was good (by Scottish standards), you could see dark cylinders moving slowly through the cold seawater. I’ve never grown out of the fear of submarines, but the house held hidden dangers of its own. I was playing in the garden. Making a pile of gravel. The house had been arsoned before we moved in, so the gutters full of gravel also contained all the glass from the shattered windows. Not that I noticed. I was grabbing handfuls, piling it up, until I registered I was bleeding from dozens of tiny cuts. I took myself up to my parents’ bedroom and triumphantly held out my hands to them. It didn’t hurt.

A fisherman on a jetty shouted at me for dropping a stone in the sea and disturbing the fish.

“I want to cut him in half,” I said, and I can still clearly see the satisfying image in my mind, of a man sliced in two like a pink salmon.

Sugar, spice, and murder fantasies. There’s a whimsical grotesquery to childhood I think we’re programmed to erase. Perhaps it’s only free to come out when we’ve been dosed up by friendly anaesthetists without names.

I haven’t blogged properly for a while, partly because I’m wrapped up in The Mighty Healer, and partly because the boyfriend and I have both been varying shades of ill, ill, ill, for several months. Nothing sucks up time like hospital visits and bedrest.

Here in the UK, the general election is about to topple onto our heads. That means blanket coverage of men in bad suits shouting at each other about the NHS, not because they care that it’s circling the drain, but because winning debates is more fun than losing them. After a while, all the chatter ceases to sound like real life and enters Pink Floyd video territory, particularly when Cameron gets a bit sweaty and you sit there hoping his rubber mask will slither off to reveal the circuitry within.

So I want to share something fun.

Karolyn Gehrig is a queer disabled artist from Los Angeles. Like me, she has a connective tissue disorder and spends a lot of time traipsing in and out of hospital. To pick herself up, Karolyn started taking selfies in a medical setting, tagging them #HospitalGlam.

They’re gorgeous.

“I started posting #hospitalglam because it was frustrating to me that every time I got sicker I’d disappear from my commitments and then feel shy about explaining where I’d been when I knew there was absolutely nothing wrong with pursuing treatment for my disabling chronic illnesses.”

The idea struck a chord. With the success of the #HospitalGlam hashtag on Twitter, Karolyn set up a Tumblr for fellow patients to submit their own selfies, giving them something to do in those doldrum waiting rooms, and winning back a bit of self-esteem at the same time. Any gender, any illness, artistic or silly, made-up or fed up.

“There are few places where one feels less empowered and beautiful than in a medical setting, and it’s not just because of that awful beige color the walls in doctor’s offices are always painted. Most humans are fortunate to only associate doctors with tedious time spent in waiting rooms and an annual check up. For those of us who are chronically ill, however, the experience of spending as much time as we do in medical settings becomes a spin-cycle of misery, depression, and – often – questioning our own sanity. […] Being disabled in an able-bodied society quickly became a source of shame and frustration.”

Being chronically ill isn’t as glamorous as the brochure made it out to be. The invisibility Karolyn talked about – self-inflicted or otherwise – has an accumulative effect on the psyche. That’s on top of the media’s relentless commodification of the body, where presentability is bound up in youth, health, and unattainable conventional beauty. That’s why it’s so important to see images of people with chronic illnesses and disabilities where a) they’re in control, and b) pity porn, or the godawful ‘inspiration’, doesn’t come into it.

Model Melanie Gaydos will be familiar from Rammstein and Die Antwoord videos. Here, she talks about growing up with Ectodermal Dysplasia and bringing her individuality to the modelling world.

“When I go on a photo shoot, if there’s other industry models there, they normally don’t really know what to make of me. And they’re usually like, ‘What the fuck is this?’ […] I love modelling. A time for me to be completely open. I guess it’s kind of like a therapeutic process for me.”

#HospitalGlam is a weapon for those of us with chronic illnesses. With it, we reassert our individuality, regain our playfulness, and have a ‘frivolous’ place to escape to when all else might seem out of control. It is therapeutic. And it’s fun. And all the while people ask what the fuck, we know it’s needed.

Today is Rare Disease Day, an international day of awareness-raising activities under the slogan “Join Together for Better Care”. Yesterday, the House of Commons held a debate on the effects of welfare cuts on the sick and disabled. As you can see, it was teeming with people who care.

While the debate dribbled on, I read a discussion on one of the Marfan Syndrome social forums. A man was expecting the birth of his son, and wanted to know how he could bring the boy up to see Marfans in a positive – or at least neutral – light. A Marf parent has a 50% chance of passing the mutated gene onto their child. If his son isn’t born with the syndrome, he will still experience the fallout of strangers’ reactions to his father. Which, let me tell you from experience, will not be the stuff gleaming Liberal utopian dreams are made on.

This had me thinking. If I could go back in time to offer my child-self advice on living with disability, what would I say?

Anyone with a rare disease or disorder will be well-acquainted with the word ‘awkward’. It usually arrives wearing a friendly mask: mild teasing when you explain you can’t help your colleague move their heavy desk, or the tutting of your fellow Girl Guides when you faint on the way to the world’s oldest toy shop and have to lie panting on the Oxford Circus floor tiles, watching commuters’ feet hurrying by.

Disability is just as much a social challenge as it is physical. You learn you are not simply inconveniencing people – you are the inconvenience.

I grew up before the Internet was permanently glued to the palms of our hands. When I eventually got a modem and an hour each weekend to play on it, it didn’t occur to me to look for advocacy groups, support services, or other people like me. My fear was that I would type ‘Marfans’ into the search field and find nothing but derision and cluelessness, as I had in real life. “If it was really that bad,” my PE teacher said when I tried to explain that trampoline + weak connective tissue = disaster, “I would have heard of it”. Then she made me get on the damn trampoline.

It took me years to gather the courage to seek out other Marfs online. When I did, I realised how valuable support groups are for those of us with rare disorders, and not merely in a fluffy, join-hands-and-sway-to-the-folk-guitar way.

“If you ever get a fuzzy black curtain descending over one side of your vision, drop what you’re doing and run to A&E – your retinas are detaching “ Good to know, yes? Or: “Get a medical alert bracelet. If you’re on the floor with a collapsed lung, you won’t be able to talk.”

Nobody had told me. I was so used to explaining the little I knew about my condition to my own doctors, to being the star attraction for medical students, to fielding gigantic questions from people in power such as the DWP employee who asked, “So, can you just, like, explain to me what this thing is?“. I had been trained to believe that even if quietly soldiering on put me in danger, at least it meant I wasn’t getting in anyone’s way.

The social model of disability says that disability is caused by the way society is organised, rather than by a person’s impairment or difference. When barriers are removed, disabled people can be independent and equal in society, with choice and control over their own lives. – Scope.

Napoleon is a hardworking taxpayer and has no time for your nonsense.

Able-bodied or otherwise, we are brought up knowing the archetypes: The Inspiring Cripple, The Grateful Invalid. Strivers and Skivers. The poisonous leftovers of the Victorian Deserving/Undeserving dichotomy. I have said before that to discuss these issues one has to cloak oneself in charm and detachment, because if there’s anything those in favour of welfare cuts can’t stand, it’s the bolshie sick. Perhaps it reminds them of the uncomfortable old adage that a society is only as civilised as its treatment of the vulnerable.

So, on Rare Disease Day, when I like to take stock of my network of support, and the possibilities of the future, I find myself looking at my country and not feeling terribly wanted. Nevertheless, I still have sufficient self-esteem to know that if I could go back and tell my child-self anything, it would be that disability does not equal inferiority. It doesn’t, despite the strenuous efforts of those more fortunate than you: people who may be elected officials, or wearing ATOS lanyards, or just little girls impatient to get to the toy shop before it closes.

There’s been a ripple through the Marfan community this week. Some American comedians I’ve vaguely heard of (my world is muffled by nineteenth century poet’s letters to their dentists) have taken it upon themselves to mention Marfan syndrome in their latest film, The Internship.

Instead of using their immense media power to spread lifesaving information to the suspected thousands of people who don’t know they have Marfans, Vince Vaughn and Will Ferrell decided it would be easier to throw this out there, apropos of nothing:

The National Marfan Foundation have released a statement urging the film’s producers to use this opportunity to spread the word about this often fatal disorder, and also pointing out the obvious: it was a stupid thing to say. Reactions from individual Marfs have ranged from “I’ve heard worse” to this, which reads like a punch in the gut:

A Virginia man, who lost his two-year-old son to Marfan syndrome in 2011, wrote that he was “extremely upset with the lack of taste, concern and respect concerning this disorder.”

The blogger Maya, also known as MarfMom, is, as always, joyful and positive, and has written about the film. She rightly thinks we ought to use this opportunity to educate, because the danger of the line is that it spreads misinformation. Half of people with Marfans don’t know they’ve got it. They go without medication, take part in dangerous activities, and may not find out until they’re in the back of an ambulance. Accurate information in the public eye is vital.

‘Big man’s disease’? Marfan syndrome affects men and women equally. ‘Giant killer’? Not all Marfs are exceptionally tall, and even so, they tend to be thin or unmuscular. In fact, if I had to pick a mythical creature to represent Marfans, it would be the willowy elves from Lord of The Rings. They’re long, they’re languid, and they’re sick to the back teeth of orcs crawling out of the woodwork. And what is this ableist obsession with fantasy monikers anyway? Giants, dwarves, monsters. Anything but human.

Elfking Thranduil thinks your attitude stinks.

Handy rule of thumb: If you don’t have a disability, don’t make jokes about it.

I’m going to have that printed on little glitzy flashcards to make it all the more memorable, because although it’s basic human decency, some people still struggle with it.

No.

All the Marfs I know make light of our health among ourselves, our friends, and families. Laughter is useful, particularly when – and this actually happened to me – a saleswoman earnestly informs you your incurable illness will clear up if you just drink enough aloe vera juice.

But if you don’t inhabit that sphere, you can’t assume what people’s thresholds are. You can’t assume what people are just about managing to cope with, what their history is, who they’ve lost.

Another thing Vaughn and Ferrell might be unaware of is that people with a long-term health conditions are strongly encouraged to keep their feelings to themselves. Classic derailing: “It’s only a joke, don’t take it so personally, no-one will take you seriously when you’re angry”. If you’re going to speak out about ableism you must cloak yourself in charm and detachment, as if this wasn’t your everyday life being discussed. Heaven forbid you become one of those frightening party-pooper militants who lurk in drains with Pennywise the clown.

Yes, it’s just a weak joke in a film unlikely to go down in cinematic history. But there will be kids who’ll suffer in school because of this. There will be adults who’ve lost a child or a parent or a friend who will have to smile politely at jokes like this from colleagues, strangers, and even friends who, because they’ve seen it in a mainstream film, think it’s harmless behaviour.

They say that one passion leads to another. Long before I discovered Dante Gabriel Rossetti, I lived in Gormenghast.

Between school bullies, kidney infections, and the oncoming Iraq war (which, I’d somehow convinced myself, was my fault), the year 2000 was a dismal time to be fourteen. But when the BBC released a four-part adaptation of Gormenghast in time for the Millennium, something shifted. From my hospital bed, I imagined the mauve peaks and crumbling spires of the castle on the horizon. I stopped doing my homework. Mervyn Peake’s Machiavellian fantasy was a safe place to escape to.

I never grew out of it. At my first University graduation, seeing the professors traipsing down the aisles in their gowns and mortar boards, I whispered excitedly to the boy next to me: “This is just like Gormenghast!” He had no idea what I was on about.

Years pass. We grow up, our tastes evolve. I fell out with high fantasy, fell into the nineteenth century. But, over a decade after my first encounter with Gormenghast, thumbing through my paperback trilogy, something sounded familiar…

“A girl of about fifteen with long, rather wild black hair. She was gauche in movement and, in a sense, ugly of face, but with how small a twist might she not suddenly have become beautiful. Her sullen mouth was full and rich – her eyes smouldered. A yellow scarf hung loosely around her neck. Her shapeless dress was a flaming red. For all the straightness of her back she walked with a slouch.”

Oh, hello, Jane Morris.

For a fourteen-year-old reader, Lady Fuchsia Groan is an easy character to relate and aspire to. Living in isolation where ‘the halls, towers, the rooms of Gormenghast were of another planet’, her response to most things is to run away to her dark attic of storybooks and paintings. She is a petulant child playing Ophelia and Juliet, dying to fall headlong into a world of chivalric romance and adventure.

Fuchsia – in Peake’s own illustrations and his text – has unmistakable similarities to Rossetti’s Jane. Like La Pia, Fuchsia glowers with the lethargic energy of someone who wants to be somewhere else but isn’t sure where. Her unkempt hair and pronounced features give her the ‘unpretty’ Pre-Raphaelite beauty the Victorians were so bothered by. Jane was considered unfortunately unattractive by many. Fuchsia, too.

There are Pre-Raphaelite echoes in every corner of Gormenghast. Maybe it’s the meeting of the Gothic and the Chivalric, the tragic and the absurd, or Peake’s own network of literary sources including Lewis Carol and The Brothers Grimm. Peake’s childhood in China and later studies at the Royal Academy gave his work a sense of ancientness and the exotic that reminds me of Holman Hunt’s picking and choosing of historical and cultural details. You can see it in The Hall of The Bright Carvings and the almost Tibetan descriptions of the endless corridors and slanting roofs of the castle.

Mervyn, acting casual

As a war artist in the 1940s, Peake saw terrible scenes of human cruelty in the rubble of the bombsites and the concentration camps. Perhaps it was only natural to head for the dusty safety of the past.

The BBC adaptation – which I realise is not to every Peake-purist’s taste – is funhouse mirror Pre-Raphaelitism. Nature is vast and unfathomable. Steerpike wheedles his way into Fuchsia’s favour by claiming to be “like the knights of old, your ladyship” only to find he can’t possibly live up to Fuchsia’s fantasies. In John Constable’s later stage show, Fuchsia is even given red hair. (Actual audience comment: “This is horrible. They said it was fantasy. It’s nothing like Harry Potter at all.”)

The BBC costumes are luxuriant. Fuchsia starts off as a teenager in a loose red velvet dress embroidered with stars. As she gets older and sadder, her outfits become heavier, more stiffly structured, until she is dragged down into the foaming floodwaters like Ophelia, leaving flowers in her wake.

The costume department referred to some of the same sources the Pre-Raphaelites did – Velázquez and Botticelli – resulting in voluminous layers of fabric and detail (even hazelnuts as buttons!) like a mad dressing-up session in a museum vault. Actress Neve McIntosh would gain two inches in height after taking off Fuchsia’s weighty gowns. Rossetti, with his reams of fabric cluttering up the house, would have loved it.

Excuse the poor quality photograph, but wouldn’t Rossetti have made a great job of this still from the film as a painting? Minus the prosthetic chin.

I wonder if I would have reacted so strongly to the Pre-Raphaelites had I not experienced Gormenghast so young. One good thing leads on to another. What’s next?

Wellcome is my favourite museum. If you have the slightest interest in wunderkammer, it’s a playground. The perfect balance of the medical, the historical, the scientific and the artistic, lovingly founded by Victorian philanthropist Henry Wellcome. Look upon his facial hair and tremble.

Science museums can feel unfriendly to artsy types, but at the Wellcome, the two disciplines interact. Upstairs, in the cool white Medicine Now room, slides of organs are displayed alongside barmy art (there’s a giant purple jellybaby as a metaphor for human cloning). Downstairs, in the darker, more anthropological Medicine Man room, you’ll find a wall of antique forceps and some beautifully detailed glass eyes which could easily be items of jewellery or sculpture. Plus, there’s a Bosch painting, and everyone loves a good Bosch.

But I think what I love the most about the Wellcome Collection is that, in a manner of speaking, I’m in it.

Connective tissue holds all parts of the body together and helps control how the body grows. Because connective tissue is found throughout the body, Marfan syndrome features can occur in many different parts of the body.

Marfan syndrome features are most often found in the heart, blood vessels, bones, joints, and eyes. Sometimes the lungs and skin are also affected. Marfan syndrome does not affect intelligence.

Specifically, Marfans is caused by a kink in the fifteenth chromosome. So imagine the surreal excitement I felt when I turned a corner in the Wellcome Collection and came across this:

There it is. The Human Genome Project, chapter 15, subheading ‘Verity’s Wonky Genes’. I took it from the shelf with both hands. Buried amongst the reams and reams of baffling code inside was the string of glyphs that spelled out Marfan Syndrome.

Only one in five-thousand people have Marfans. The syndrome will generally make you around six feet tall and willowy in build, with exceptionally long, spidery fingers and toes. You may have a curvature of the spine or an uneven ribcage, and you can probably bend your thumbs into strange angles. Abraham Lincoln probably had it, as did Jonathan Larson, Joey Ramone, and, I strongly suspect, Lux Interior of The Cramps.

Marfans can affect you in all sorts of strange, annoying, sometimes life-threatening ways. Individual Marfs differ. As for me, I’m well looked-after by good doctors. I pace myself, I watch my diet and try not to be a stubborn ass when it comes to clinging to the barrier at Morrissey concerts or vigorous charity shopping the weekend after minor heart surgery. (Although holding hands with Morrissey and acquiring an antique nursing chair for £10 were worth the resulting drama).

It’s an ambition of mine to get Marfans into the Wellcome more prominently. Short of standing in the entrance hall with a sign on me, I don’t know how to raise awareness. I’m not quite ready to donate my hands. But it’s a syndrome that really lends itself to art. Maybe I can use my nonexistent artistic ability to chop up my MRIs in a nice lightbox, or draw an Edward Gorey-esque bunch of spidery fingers. Or, better still, persuade someone who actually knows what they’re doing to put Marfans in front of the lens, like Alexa Wright’s ‘After Image’ series.